Long-term follow-up of patients with familial adenomatous polyposis undergoing pancreaticoduodenal surgery

Abstract

Adenomatous polyps and adenocarcinomas of the periampullary region are the most common upper gastrointestinal neoplasms encountered in familial adenomatous polyposis (FAP) patients. Tumors arising from the liver, biliary tract, and pancreas have also been reported. The purpose of this study was to review the clinical outcome of FAP patients after pancreaticoduodenal surgery for periampullary neoplasms. Of the 61 individuals participating in our prospective FAP registry, 8 underwent surgical resection of periampullary neoplasms between 1987 and 1998. The charts of these individuals were reviewed for clinical indications, type of pancreaticoduodenal surgery, postoperative complications, and outcome. Of the 8 patients identified, 7 had pancreaticoduodenectomy and 1 had duodenotomy with ampullectomy. The indications for surgery were periampullary cancer (3), severe dysplasia within a duodenal villous tumor (4), and solid-pseudopapillary tumor of the pancreas (1). At the time of pancreaticoduodenal surgery, patients ranged in age from 29-65 years, and all but one had undergone colorectal surgery, on average 16 years beforehand. Pancreatic ascites after a pylorus-sparing pancreaticoduodenectomy was the only surgical complication. At a median follow-up of 70.5 months (range 37-162), 2 patients had died, neither from their periampullary neoplasm. The patient treated by local excision subsequently developed gastric cancer arising from a polyp and went on to gastrectomy. Another patient developed confluent benign jejunal adenomas just beyond the gastroenteric anastomosis almost 12 years after pancreaticoduodenectomy for severe dysplasia of a duodenal villous adenoma. Pancreaticoduodenectomy is a safe and appropriate surgical option for FAP patients with duodenal villous tumors containing severe dysplasia or carcinoma. Postoperative morbidity was minimal and there was no perioperative mortality. Good long-term prognosis can be expected in completely resected patients although subsequent proliferative and/or neoplastic lesions may still be detected in the gastrointestinal tract with prolonged follow-up.